Pediatric Cardiology

, Volume 40, Issue 1, pp 147–153 | Cite as

Examining the Utility of Coronary Artery Lack of Tapering and Perivascular Brightness in Incomplete Kawasaki Disease

  • Edon J. RabinowitzEmail author
  • Lorry G. Rubin
  • Kinjal Desai
  • Denise A. Hayes
  • Aykut Tugertimur
  • Elena N. Kwon
  • Preeta Dhanantwari
  • Nilanjana Misra
  • Guillaume Stoffels
  • Andrew D. Blaufox
  • Elizabeth Mitchell
Original Article



In 2017, the AHA published revised guidelines for the diagnosis of Kawasaki disease (KD). In the absence of compelling data supporting or refuting the utility of lack of tapering (LT) and perivascular brightness (PB), expert panel consensus removed LT and PB from consideration. We hypothesize that LT and PB are unreliable, subjective findings, non-specific to KD, which can be seen in systemic febrile illnesses without KD and in normal controls.


We performed a single-center retrospective study from 1/2008 to 12/2016. De-identified coronary artery (CA) echocardiographic clips from patients 0–10 years old were interpreted blindly by six pediatric cardiologists. Subjects were grouped as follows: (1) healthy: afebrile with benign murmur, (2) KD: IVIG treatment, 4–5 clinical criteria at presentation, (3) incomplete KD (iKD): IVIG, 1–3 clinical criteria, (4) Febrile: ≥3 days of fever, no IVIG, KD not suspected. The presence or absence of LT and PB was recorded. Inter-rater and intra-rater reliabilities were analyzed using intra-class correlation coefficient, Fleiss’ Kappa and Cohen’s Kappa coefficients.


We interpreted 117 echocardiograms from healthy (27), KD (30), iKD (32), and febrile (28) subjects. Analysis showed moderate agreement in CA z score measurements. LT and PB were observed by most readers in control groups. LT exhibited fair inter-reader agreement (reliability coefficient 0.36) and PB slight inter-reader agreement (reliability coefficient 0.13). Intra-rater reliability was inconsistent for both parameters.


LT and PB are subjective, poorly reproducible features that can be seen in febrile patients without KD and in healthy children.


Incomplete Kawasaki disease Kawasaki disease Lack of tapering Perivascular brightness Coronary vessel disease Acquired heart disease 



Kawasaki disease


Incomplete Kawasaki disease


Lack of tapering


Perivascular brightness


Coronary artery


Intravenous immunoglobulins




Left main coronary artery


Left anterior descending artery


Right coronary artery


Body surface area


Compliance with Ethical Standards

Conflict of interest

All authors declare that they have no conflict of interest relevant to this article to disclose.

Ethical Approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed Consent

A waiver of informed consent was obtained from the Northwell Health institutional review board.


  1. 1.
    Son MBF, Newburger JW (2013) Kawasaki disease. Pediatr Rev 34(4):151–162CrossRefGoogle Scholar
  2. 2.
    Dajani AS, Taubert KA, Gerber MA et al (1993) Diagnosis and therapy of Kawasaki disease in children. Circulation 87(5):1776–1780CrossRefGoogle Scholar
  3. 3.
    Kato H, Sugimura T, Akagi T et al (1996) Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients. Circulation 94(6):1379–1385CrossRefGoogle Scholar
  4. 4.
    Fujiwara H, Hamashima Y (1978) Pathology of the heart in Kawasaki disease. Pediatrics 61(1):100–107Google Scholar
  5. 5.
    Yutani C, Okano K, Kamiya T et al (1980) Histopathological study on right endomyocardial biopsy of Kawasaki disease. Br Heart J 43(5):589–592CrossRefGoogle Scholar
  6. 6.
    McCrindle BW, Rowley AH, Newburger JW et al (2017) Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 135(17):e927–e999CrossRefGoogle Scholar
  7. 7.
    Newburger JW, Takahashi M, Gerber MA et al (2004) Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 114(6):1708–1733CrossRefGoogle Scholar
  8. 8.
    Baer AZ, Rubin LG, Shapiro CA et al (2006) Prevalence of coronary artery lesions on the initial echocardiogram in Kawasaki syndrome. Arch Pediatr Adolesc Med 160(7):686–690CrossRefGoogle Scholar
  9. 9.
    Minich LL, Sleeper LA, Atz AM et al (2007) Delayed diagnosis of Kawasaki disease: what are the risk factors? Pediatrics 120(6):e1434–e1440CrossRefGoogle Scholar
  10. 10.
    Sonobe T, Kiyosawa N, Tsuchiya K et al (2007) Prevalence of coronary artery abnormality in incomplete Kawasaki disease. Pediatr Int 49(4):421–426CrossRefGoogle Scholar
  11. 11.
    Wyman L (2009) Echocardiography in pediatric and congenital heart disease: from fetus to adult. West Sussex, HobokenGoogle Scholar
  12. 12.
    Landis JR, Koch GG (1977) The measurement of observer agreement for categorical data. Biometrics 33(1):159–174CrossRefGoogle Scholar
  13. 13.
    Newburger JW, Taubert KA, Shulman ST et al (2003) Summary and abstracts of the seventh international Kawasaki disease symposium: December 4–7, 2001, Hakone, Japan. Pediatr Res 53:153Google Scholar
  14. 14.
    Yu JJ, Jang WS, Ko HK et al (2011) Perivascular brightness of coronary arteries in Kawasaki disease. J Pediatr 159(3):454–457 e451CrossRefGoogle Scholar
  15. 15.
    Abe O, Karasawa K, Hirano M et al (2010) Quantitative evaluation of coronary artery wall echogenicity by integrated backscatter analysis in Kawasaki disease. J Am Soc Echocardiogr 23(9):938–942CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Edon J. Rabinowitz
    • 1
    Email author
  • Lorry G. Rubin
    • 2
  • Kinjal Desai
    • 3
  • Denise A. Hayes
    • 1
  • Aykut Tugertimur
    • 1
  • Elena N. Kwon
    • 1
  • Preeta Dhanantwari
    • 1
  • Nilanjana Misra
    • 1
  • Guillaume Stoffels
    • 4
  • Andrew D. Blaufox
    • 1
  • Elizabeth Mitchell
    • 1
  1. 1.Division of Pediatric CardiologyCohen Children’s Medical Center of New York – Hofstra Northwell School of MedicineNew Hyde ParkUSA
  2. 2.Division of Pediatric Infectious DiseaseCohen Children’s Medical Center of New York – Hofstra Northwell School of MedicineNew Hyde ParkUSA
  3. 3.Department of PediatricsCohen Children’s Medical Center of New York – Hofstra Northwell School of MedicineNew Hyde ParkUSA
  4. 4.Feinstein Institute Department of BiostatisticsCohen Children’s Medical Center of New York – Hofstra Northwell School of MedicineNew Hyde ParkUSA

Personalised recommendations